Veterinary Dermatology 2005, 16, 239252

Clinical, histopathological and immunological characteristics

of exfoliative cutaneous lupus erythematosus in 25 German
short-haired pointers

Blackwell Publishing, Ltd.

SHARON L. BRYDEN*, STEPHEN D. WHITE, STANLEY M. DUNSTON,

AMANDA K. BURROWS* and THIERRY OLIVRY
*Murdoch University Veterinary Hospital, Division of Health Sciences, School of Veterinary and Biomedical
Science, Murdoch University, Murdoch, Western Australia 6150, Australia
Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh,
North CA 27606, USA
Department of Medicine and Epidemiology, School of Veterinary Medicine, University of California, Davis,
CA 95616, USA
(Received 14 October 2004; accepted 15 June 2005)

Abstract Clinical, histopathological and immunological features of exfoliative cutaneous lupus erythematosus,
an uncommon generalized exfoliative dermatitis occurring exclusively in German short-haired pointers, were
characterized in 25 dogs. The disease affects young adult dogs and its familial incidence strongly suggests a hereditary origin. Lesions were characterized by scaling and alopecia affecting 100 (25/25) and 76% (19/25) of dogs,
respectively. Follicular casts were present in 28% (7/25) of dogs. The muzzle, pinnae and dorsum were typically
affected. Generalized skin lesions were described in 52% (13/25) of dogs. Systemic signs of pain and lameness
affected several dogs. Anaemia and thrombocytopenia were detected in several dogs with a more severe clinical
phenotype. The most common histopathological features were hyperkeratosis and a lymphocytic interface dermatitis. Direct immunostaining revealed IgG deposition in the epidermal and follicular basement membrane of
100 (19 / 19) and 41% (7 / 17) of dogs, respectively. Circulating antifollicular and antisebaceous gland IgG antibodies were demonstrated by indirect immunostaining in 57% (4/7) of dogs. This disease usually responds poorly
to immunosuppressive therapy and it has a guarded prognosis. Where outcome was recorded, 85% (10/12) of
dogs were euthanased due to either a failure to respond to, or complications associated with, immunomodulatory
therapy. Two affected dogs are in remission and maintained on immunomodulatory dosages of prednisolone. This
study demonstrates the existence of a cellular and humoral immune response directed against the epidermal
basement membrane of dogs with exfoliative cutaneous lupus erythematosus. Additional studies are required to
further characterize the immunological pathogenesis of this disease.

IN TRO D U CT ION
An acquired, generalized exfoliative dermatitis originally reported as hereditary lupoid dermatosis of the
German short-haired pointer (GSP)1 has been described
as unique to this breed. It has previously been reported
in European, American and Australian GSP dogs.17
Dogs clinically affected with this skin disease are
reportedly young adults between 6 months and 2.75 years
at the age of onset.16 These dogs develop scaling that
initially affects the face, pinnae and dorsum and which
progresses to a more generalized distribution. 17
Peripheral lymphadenopathy and, less often, pyrexia
have been reported.17 As affected subjects exhibit a
lymphocytic interface dermatitis similar to lupus-specific
dermatoses of humans, it has recently been proposed

Correspondence: Sharon Bryden, Murdoch University Veterinary

Hospital, Division of Health Sciences, School of Veterinary and
Biomedical Science, Murdoch University, Murdoch, Western
Australia 6150, Australia. E-mail: sbryden@murdoch.edu.au
2005 European Society of Veterinary Dermatology

to call this syndrome exfoliative cutaneous lupus

erythematosus (ECLE) of GSP.2
The pathogenesis of ECLE is poorly understood but
the recognition of this disease exclusively in GSP and
its familial incidence strongly suggest a hereditary
origin.1,6 Histological examination of skin biopsy
specimens from affected dogs reportedly reveals a
lymphocyte-rich interface dermatitis and superficial
mural folliculitis as the dominant histopathological
pattern.2 IgG has been detected by direct immunofluorescent testing at the epidermal and infundibular basement
membrane in the majority of dogs evaluated.2 Circulating
basement membrane or sebaceous gland-specific autoantibodies or antinuclear antibodies, however, have
not been detected previously in affected dogs.2
Successful management of ECLE has proven frustrating with a lack of consistent response to individual
therapies. 27 Topical antiseborrhoeic shampoo and
humectant application, oral fatty acid supplementation
and oral tetracycline and niacinamide administration
have all produced transient improvement but have failed
to achieve long-term remission.27 Immunosuppressive
239

240

SL Bryden et al.

therapy using prednisolone has been used successfully

to treat ECLE in three GSP siblings, in combination
with azathioprine in one dog6 The prognosis for this
disease is poor because of a failure to respond to, or
complications associated with treatment.27
In this study we sought to define further the genetic
background and clinical features, as well as the management and prognosis, of dogs with ECLE by detailed
retrospective evaluation of available case records.
Pedigree information, where available, was accessed
either from the medical records or from owners of the
affected dogs. Interpretation of the pedigree data was
completed by one of the authors (SW). In addition, the
histopathological and immunological features of ECLE
were further studied by obtaining archived paraffinembedded skin biopsy specimens and stored frozen
serum samples from affected individuals and performing histological and direct and indirect immunofluorescence and immunohistochemical studies on all
available material. These studies were completed by the
authors (SB and TO) at North Carolina State University, North Carolina.

Table 1. Case material and investigations performed

MATERIALS AND ME T HODS

Q Questionnaire; MR Medical Record; + Material

available for investigation; NT Investigation not performed;
Histo Histopathology; DIF Direct Immunofluorescence;
IIF Indirect immunofluorescence; IHC Immunohistochemistry.

Case material
Case material was included from 25 GSP dogs from
veterinary private practices and referral institutions in
the USA, UK and Australia in which a clinical and
histopathological diagnosis of ECLE had been made by
the referring veterinarian. A retrospective evaluation
of the patient records (seven dogs) or a questionnaire
completed by the referring veterinarian (18 dogs) was
used to collect information on the history and clinical
signs from all 25 dogs, and treatment from 23 dogs.
Long-term follow-up was available for 13 dogs. Pedigree information was available for 15 of the 25 dogs
(see Table 1). In addition, pedigree information was
included from another four affected dogs not included
in this study.

Histological examination
Skin biopsy material was available for 23 dogs (see
Table 1). Routinely processed, paraffin-embedded blocks
were sectioned and stained with haematoxylin & eosin.
Histological features of hyperkeratosis, interface dermatitis (defined as basal vacuolation, blurring of basement membrane zone and basal apoptosis), apoptosis
within the upper epidermis, lymphocytic infiltration of
the upper and lower epidermis and superficial dermis,
interface mural folliculitis and sebaceous gland and
sweat gland inflammatory infiltrate were evaluated.
Microscopic features of each section were scored
semiquantitatively as follows: intensity absent (0), mild
(1), moderate (2) or marked (3) and extent absent (0),
focal (1), multifocal (2) or diffuse (3). The average score
for each dog was then calculated for each parameter.
The number of dogs with a score fulfilling the criteria for
absent (0), mild (0.011), moderate (1.012) or marked

Case #

Q/ MR Pedigree Histo DIF IIF IHC Follow-up

1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25

Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
Q
MR
MR
MR
MR
MR
MR
MR

+
+
+
+
+
+
+
+
+
+
+
+
+
NT
NT
NT
NT
NT
NT
NT
NT
NT
+
+
NT

+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
+
NT
+
NT

+
NT
NT
+
+
+
+
+
+
+
+
+
+
+
NT
NT
+
+
NT
+
NT
+
+
+
+

+
NT
NT
NT
NT
NT
NT
NT
NT
NT
NT
+
+
NT
NT
NT
NT
NT
+
+
+
NT
NT
+
NT

+
+
+
+
+
+
+
NT
+
+
+
+
+
+
+
+
+
+
+
+
NT
NT
+
+
NT

+
NT
NT
+
NT
+
+
NT
NT
+
NT
NT
+
NT
+
+
NT
NT
+
+
+
NT
+
+
NT

(2.013) was then calculated (see Table 4). The absence

of sebaceous glands was also recorded for each section.
Skin biopsy specimens were available from 23 dogs,
with one dog (case 10) sampled twice at an interval of
3 months. Ninety-nine sections were evaluated in total.

Direct immunofluorescence
In situ deposition of IgG, IgA and IgM antibodies and
activated complement (C3 component) was detected
by direct immunofluorescence (IF) testing of paraffinembedded sections from 19 dogs (see Table 1).
These were deparaffinized, rehydrated and digested
with 0.1% trypsin (#T-8128, Sigma Chemical Company, St Louis, MO, USA) for 35 min at 37 C for antigen retrieval. After proteolysis, the sections were rinsed
in phosphate buffered saline (PBS) and blocked with
1% newborn calf serum (#N-4762, Sigma Chemical
Company). The sections were incubated for 30 min at
room temperature with either goat antidog IgA (Fitc)
(A40104F Bethyl Laboratories, Montgomery, TX,
USA), goat antidog IgM (Fitc) (#A40116F Bethyl
Laboratories), goat antidog C3 (Bethyl Laboratories)
at 1 : 40 or rabbit antidog IgG (Fitc) (#672081 ICN
Biomedical, Aurora, OH, USA) at 1 : 60. PBS replacement of antibody and normal canine spleen served as
negative and positive controls, respectively. After further rinsing with PBS the sections were counterstained
with Evans Blue (#E013, Sigma Chemical Company),
2 drops per 500 mLs for 10 s and mounted with
Vectashield DAPI (#H 1200, Vector Laboratories,
Burlingame, CA, USA).

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Characteristics of exfoliative cutaneous lupus erythematosus

241

Seventy-six biopsy specimens from 19 dogs were

evaluated using an epifluorescence microscope. Immunofluorescence was recorded as either present or absent at
the basement membrane zone, sebaceous gland or hair
follicle level for each biopsy section. In addition, the
extent of the fluorescence as focal, multifocal or continuous and a qualitative assessment of either a fine or
thick deposition were recorded.

Lymphocytic infiltrate was recorded for the upper

and lower epidermis, superficial dermis, follicular
infundibulum, follicle below the level of the infundibulum, sebaceous and sweat glands. The extent of the
infiltrate was recorded as absent (0) focal (1), multifocal (2) or diffuse (3) and the intensity as absent (0), mild
(1), moderate (2) or marked (3).

Indirect immunofluorescence

R E SU LT S

Detection of circulating basement membrane, hair

follicle or sebaceous gland specific autoantibodies was
achieved with an indirect immunofluorescence method
using normal canine intact lip, normal canine salt-split
lip and normal canine haired skin sections was
performed in seven dogs (see Table 1).8 These were
immersed in acetone for 10 min and after rinsing in
PBS were blocked with 1% newborn calf serum (#N4762, Sigma Chemical Company) for 30 min in a moist
chamber. The newborn calf serum was drained and
each patients serum was applied for 1 h at 1 : 10, 1 : 50
and 1 : 100 dilutions at room temperature. For a negative control, the patients serum was substituted with
normal canine serum (NCS#3) and PBS. For a positive
control serum from a dog with confirmed epidermolysis bullosa was utilized.8 The sections were rinsed twice
in PBS for 5 min then the secondary antibody, rabbit
antidog IgG (#672081 ICN Biomedical, Aurora, OH,
USA) was applied for 30 min in a moist chamber,
followed by PBS rinse for 5 min. The sections were
then counterstained with Evans Blue (#E013, Sigma
Chemical Company) at 2 drops per 500 mL for 15 s.
After final rinsing, sections were mounted using
Vectashield DAPI (#H 1200, Vector Laboratories).

Immunohistochemistry
Immunophenotyping of skin infiltrating mononuclear
cells was performed on unstained paraffin-embedded
skin sections in 21 dogs using the T-lymphocyte marker
CD3 to identify infiltrating lymphocytes within the
epidermis, dermis and pilosebaceous units (see Table 1).
The sections were deparaffinized and rehydrated for
10 min and after rinsing in PBS were incubated with
3% protease (#P-5147, Sigma Chemical Company) for
35 min at 37 C for antigen retrieval. After rinsing with
PBS the sections were incubated with 1% newborn calf
serum (NCS) (#N-4762, Sigma Chemical Company)
for 20 min, then the primary antibody against CD3 at
1 : 1000 was applied and incubated at room temperature for 30 min. The sections were rinsed with PBS and
the secondary antibody goat antirabbit IgG (#1000,
Vector Laboratories) at 1 : 400 was applied for 30 min
at room temperature. After rinsing with PBS the tertiary
antibody Strepavidin-HRP (Zymed, San Francisco,
CA, USA) at 1 : 400 was applied and incubated at
room temperature for 30 min. The sections were rinsed
in PBS and AEC (Substrate Kit, Biogenex, San Ramon,
CA, USA) applied, rinsed with water and counter-stained
with haematoxylin. After final rinsing the sections were
mounted.

Case material
Twenty-five dogs were affected with ECLE and of these
17 were female and eight were male. The female to male
sex ratio was approximately 2 : 1. Most of the dogs
were from the USA (19), four were from Australia and
two were from the UK. The median age of onset of
ECLE was 10 months with a range of 1.848 months.
The most prominent skin lesions in the dogs were
scaling and alopecia, which affected 25 (100%) and 19
(76%) dogs, respectively (see Table 2 and Figs 14).
Follicular casts were specifically noted in seven (28%)
dogs. Skin lesions typically affected the muzzle, pinnae
and dorsal trunk and then progressed to involve the
limbs and ventral trunk. Generalized skin lesions were
described in 13 (52%) dogs. Crusting, with or without
associated ulceration was recorded in six (24%) dogs.
In one patient (case 19), ulceration was extensive and
resulted in bacterial septicaemia. One dog (case 14)
initially presented with depigmentation and ulceration
of the planum nasale (Fig. 2). Mild pruritus was recorded
in seven (28%) dogs.
A generalized peripheral lymphadenomegaly
was reported in eight (32%) dogs. In seven (28%) dogs,
intermittent episodes of pain (manifested as back arching when standing, vocalizing, experiencing difficulties
sitting or rising or displaying an altered gait) were
reported by the owners. Three (12%) dogs experienced
intermittent pyrexia.

Laboratory evaluation
Serum biochemistry and haematological examination
was performed on six and nine dogs, respectively (see
Table 2). A mild nonregenerative anaemia was recorded
for one dog (case 20) and an unspecified anaemia was
noted in three others. Thrombocytopenia was recorded
in six dogs. In four dogs there were no relevant biochemical abnormalities, one dog had a mild decrease in
serum albumin, and another dog had a mild decrease
in serum urea. Urinalysis was performed on one dog
(case 19) and revealed mild proteinuria; however, the
urine protein creatinine ratio was 0.4 units (normal
range < 0.5 units). Sera was obtained from eight dogs
and evaluated for the presence of circulating antinuclear antibodies (ANA). In one dog (case 14) serum
revealed low levels of circulating antinuclear antibodies at a titre of 1 : 40. The sera of the remaining seven
dogs were negative.
Fine-needle aspirate material from enlarged peripheral
lymph nodes was submitted for cytological evaluation

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Onset
(m)

Country

Sex

Lesions

Distribution

10

USA

Scaling, alopecia

12

USA

Scaling, alopecia

USA

USA

Scaling, alopecia,
follicular casts
Scaling, alopecia

pinnae and muzzle initially,

then dorsum and hind limbs
pinnae and muzzle
and hind limbs
generalized

Other
tests

ANA

CBC NSA
Biochemistry NSA
NR

NR

negative

NR

NR

CBC thromobocytopenia,
lymphopenia, anaemia,
NR

NR

NR

NR

NR

USA

Scaling, alopecia,
crusting and
ulceration

pinnae and
muzzle

NR

negative

NR
NR

CBC thromobocytopenia,
lymphopenia, anaemia,
Biochemistry
mild decrease in albumin
NR
NR

6
7

7
4

USA
USA

F
M

12

USA

generalized
head and dorsum
initially then hind limbs
generalized

10

USA

10

10

USA

Scaling
Scaling, alopecia,
follicular casts
Scaling, alopecia,
follicular casts;
mild pruritus
Scaling, alopecia,
crusting
Scaling, alopecia

NR
NR

NR
NR

NR

NR

NR

NR

muzzle

NR

NR

NR

NR

pinnae, muzzle
and ventrum
muzzle, pinnae
and dorsum
initially then ventrum

pain / lameness

NR

NR

NR

11

14

USA

NR

NR

NR

NR

12

14

USA

muzzle, dorsum,
ventrum and
hind limbs
generalized

NR

NR

NR

negative

13

USA

pain / lameness

NR

NR

negative

14

36

USA

15
16

10
1.8

USA
USA

17
18

42
3

USA
USA

generalized

Concurrent
clinical findings
NR
NR
(demodicosis)
Lymphadenopathy,
pyrexia
Lymphadenopathy,
pain/lameness
Lymphadenopathy

Laboratory evaluation

planum nasale
initially;
generalized

NR

CBC mild
thrombocytopenia,
Biochemistry NSA

NR

weakly
positive
1 : 40

M
M

Scaling, alopecia,
crusting, ulceration,
erythema,
mild pruritus
Scaling, alopecia,
crusting, follicular
casts
Scaling, alopecia,
mild pruritus
Scaling, alopecia,
erythema, mild
pruritus, follicular
casts; ulceration
and depigment-ation
of planum nasale
initially
Scaling, erythema
Scaling, alopecia

generalized
generalized

NR
NR

NR
NR

negative
NR

F
F

Scaling
Scaling, alopecia

pinnae, planum nasale

generalized

Lymphadenopathy
Lymphadenopathy
pain/lameness pyrexia
NR
Lymphadenopathy

NR
NR

NR
NR

NR
NR

SL Bryden et al.

Case

242

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Table 2. Clinical and laboratory results summary

Case

Onset
(m)

Country

Sex

Lesions

Distribution

Concurrent
clinical findings

CBC thrombocytopenia,
Biochemistry NSA,
UA proteinuria, UPC

Radiographs NSA,
joint aspirates NSA,
abdominal ultrasound

negative

FNA lymph
nodes lymphoid
hyperplasia

negative

NR

NR
NR

ANA

AUST

Scaling, alopecia,
crusting, ulceration,
follicular casts

muzzle and pinnae initially

then dorsum, hind limbs
becoming generalized

20

AUST

Scaling, alopecia

muzzle and dorsum

initially then pinnae

21

10

AUST

Scaling

NR

22

AUST

Scaling, alopecia,
follicular casts

dorsum initially then

pinnae and muzzle
dorsum

Lymphopenia,
pain / lameness

CBC thrombocytopenia,
anaemia (mild
nonregenerative),
Biochemistry mild
decrease urea
CBC NSA,
Biochemistry NSA
CBC thromobocytopenia,
anaemia

23

12

UK

NR

UK

Radiographs NSA

NR

48

USA

Pain/lameness
demodicosis
Emaciated
(abandoned)

CBC NSA

25

dorsum initially
then generalized scale
pinnae, dorsum,
muzzle then generalized
muzzle initially
then generalized

NR

24

Scaling, mild
intermittent pruritus
Scaling, moderate
intermittent pruritus
Scaling, alopecia,
mild intermittent
pruritus

Radiographs
NSA, myelogram
& CSF tap NSA
Heska Topscreen

NR

ELISA

NR

NR

243

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Other
tests

19

NR Not recorded; NRA No relevant abnormalities; UA urinalysis; UPC urine protein:creatinine ratio.

Lymphadenopathy,
pain / lameness,
pyrexia, weight
loss, demodicosis
Lymphadenopathy

Laboratory evaluation

Characteristics of exfoliative cutaneous lupus erythematosus

Table 2. Continued

244

SL Bryden et al.

Figure 3. Photo: Head. Case 19. Marked scaling and alopecia

involving the muzzle, pinnae and head.

Figure 1. Photo: case 14. Multifocal areas of alopecia over the

muzzle, trunk and hind limbs. Depigmentation is visible on the
planum nasale.

Figure 4. Photo: dorsal muzzle. Case 19. Marked scaling and

alopecia involving the muzzle.

Figure 2. Photo: dorsal muzzle. Case 14. Alopecia and scaling on

the dorsal muzzle. Depigmentation is visible on the planum nasale.

in one dog (case 20) with lymphadenomegaly and

revealed lymphoid hyperplasia. Spinal radiographs
(cases 19, 20, 24), myelogram and cerebrospinal fluid
(CSF) analysis (case 22) and stifle and hock joint aspirates (case 19) were performed in dogs suffering from
intermittent pain but failed to identify any underlying
abnormality. Multiple deep skin scrapings identified
generalized demodicosis in three dogs receiving immunomodulatory therapy for ECLE.

Medical management
Information regarding medical management was
obtained retrospectively. In this light individual patient
case records often failed to specify the dose rate or the
duration of prescribed treatments and the response to
therapy. Most dogs received a combination of treatments including topical keratolytic and keratoplastic
and/or antimicrobial shampoo therapies and emollients
with oral antimicrobial and immunomodulatory agents
(see Table 3).

A number of immunomodulatory agents were prescribed either as single or combination therapy in all 23
dogs and these included oral tetracycline (or doxycycline) with niacinamide (five dogs), essential fatty acid
supplements (13 dogs), prednisolone (18 dogs), azathioprine (five dogs), retinol and synthetic retinoids (three
dogs), cyclosporin (one dog) and leflunamide (one
dog).
While there was no recorded response to therapy for
16 dogs, the response to immunomodulatory therapy
in the remaining nine dogs was, in general, poor.
Temporary or partial remission was obtained in several
dogs using a combination of essential fatty acids and
tetracycline (or doxycycline) and niacinamide but
the condition either relapsed (cases 7, 19) or failed to
improve (case 10). Similar responses were achieved
with the administration of oral retinol and retinoids
(case 7). In one dog (case 1), signs partially responded
to leflunamide with a reduction in pain but there was a
persistence of dermatological signs. Pentoxifylline,
cyclosporin, topical 0.015% triamcinolone spray (Genesis Virbac, AH, Fort Worth, TX, USA) and/or topical gentamicin and betamethasone spray (Gentocin
Topical Spray Schering AH, NSW, Australia) were
used to alleviate pruritus in two dogs (cases 11, 25).

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Characteristics of exfoliative cutaneous lupus erythematosus

245

Table 3. Treatment and Outcome Summary

Case

Treatment

Response

Outcome

Topical, antibiotics (NR), prednisolone, leflunamide

Euthanased due to cost

of leflunamide

2
3
4
5
6
7

NR
Antibiotics (cephalexin, enrofloxacin), azathioprine
Topical, antibiotics (NR), prednisolone, azathioprine
Topical, EFA, antibiotics (Clavamox)
NR
EFA, tetracycline/niacinamide, antibiotics (NR), retinol

8
9
10

Topical, EFA, antibiotics (cephalexin), prednisolone

EFA, antibiotics (cephalexin)
Topical, tetracycline/niacinamide, prednisolone,
azathioprine, retinoids, pentoxifylline, zinc, vitamin E

11

Topical, EFA, prednisolone, retinol, topical

Betamethasone spray (Gentocin)
Topical, EFA, antibiotics (cephalexin),
prednisolone, azathioprine
Topical, EFA, antibiotics (NR), prednisolone
Topical, EFA, tetracycline/niacinamide,
antibiotics (cephalexin), prednisolone

Improvement after leflunamide;

marked reduction in pain but
scaling and alopecia remained
NR; demodicosis
NR
NR
NR
NR
90% resolution within 1 month;
relapsed when withdrawn
NR
NR
temporary remission on retinoids and
topical, no response to prednisolone
azathioprine, tetracycline/niacinamide
NR

12
13
14

15
16
17
18
19

20
21
22
23
24
25

Prednisolone
Antibiotics (cephalexin), prednisolone
Antibiotics (NR)
Antibiotics (NR), prednisolone
Topical, EFA, doxycycline/niacinamide,
antibiotics (cephalexin; enrofloxacin and Clavulox when
septicaemic), prednisolone, azathioprine
Topical, antibiotics (cephalexin, Clavulox) prednisolone
Topical, antibiotics (cephalexin), prednisolone
Topical, EFA, tetracycline/niacinamide,
antibiotics (Clavulox), prednisolone
Topical, EFA, antibiotics (potentiated
sulphonamide), prednisolone
Topical, EFA, antibiotics (cephalexin), prednisolone
Topical, EFA, antibiotics (cephalexin), prednisolone,
pentoxifylline, Genesis, antihistamine
(diphenhydramine), cyclosporin

NR
NR
Euthanased
NR
Euthanased
Euthanased
NR
NR
Euthanased

NR

NR

NR

NR
improved 1 month after commencing
prednisolone, tetracycline/niacinamide
and EFA
NR
NR
NR
NR
Remission 31 m then waxing and
waning; demodicosis

Euthanased after 4 years

NR

Remission 20 m
Remission 24 m
NR

Euthanased after 2 years

NR
NR

waxing, waning, EFA, topical currently,

no response to low dose prednisolone
NR; demodicosis
waxing and waning; concurrent atopy

Currently treated

euthanased
euthanased
NR
NR
Euthanased after 3.5 years

Euthanased after 4 years

Currently treated

NR Not recorded; EFA essential fatty acids.

Oral prednisolone was administered to 18 dogs and,

where specified, dose rates ranged from 0.4 mg kg1 to
2 mg kg1 orally every 24 h. In general, lower dosages
of prednisolone were not associated with clinical
improvement whereas clinical remission was achieved
with higher dose rates in some cases. The most consistent
response to immunomodulatory therapy was achieved
with three littermates (cases 19, 20 and 21) in which
remissions of 31, 20 and 24 months, respectively, were
achieved using oral prednisolone at 2 mg kg1 q24 h in
combination with topical keratolytic/keratoplastic
and emollient therapy. The dog with the longest remission (case 19) received concurrent azathioprine at
2 mg kg1 orally every 24 h.
While there was no long-term outcome recorded for
12 dogs (see Table 1), 11 dogs were euthanased due to
either a failure to respond to, or complications associated with, immunomodulatory therapy. One dog (case
25) in this study is currently maintained on oral prednisolone at a dosage of 0.25 mg kg1 administered on

an alternate day basis in conjunction with oral essential

fatty acid supplementation. The referring veterinarian
has reported a waxing and waning of the disease course.

Pedigree information
Pedigree evaluation demonstrated that six dogs (see
Table 1) from the USA (cases 1, 2, 4, 6, 7 and 9) were
related. In addition, four other dogs with ECLE (also
from the USA) not included in this study were related
to these dogs. Figure 5 shows the familial relationship
of these 10 dogs. In addition, three other dogs (cases
19, 20, 21) from Australia were full littermates. A fourth
littermate was not affected.

Histological examination
Histological examination of H&E-stained sections of
skin biopsy specimens revealed a predominantly moderate, diffuse hyperkeratosis and moderate-to-marked,
multifocal interface dermatitis in all dogs (see Table 4 and
Fig. 6). In addition, the majority of dogs demonstrated

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246

SL Bryden et al.

Figure 5. Pedigree Chart. Key:

Square = male; Circle = female;
Red = affected (histologically confirmed);
Numbered animals correspond to the dogs
of this report listed in Tables 1, 2, 3 and 5.

Table 4. Histopathology Results

Absent (0)
Mild/focal (0.11)
Moderate/multifocal (1.012)
Marked/diffuse (2.013)

Interface

Intensity

Extent

Intensity

Extent

Intensity

Extent

Intensity

Extent

0
3
12
9

0
0
11
3

0
2
11
11

0
0
18
6

7
15
2
0

7
13
4
0

4
18
2
0

4
11
9
0

Lymphocytic
infiltrate led

Absent (0)
Mild/focal (0.11)
Moderate/multifocal (1.012)
Marked/diffuse (2.013)

Apoptosis

Lymphocytic
infiltrate sd

Interface above
infundibulum

Interface below
infundibulum

Intensity

Extent

Intensity

Extent

Intensity

Extent

Intensity

Extent

0
3
13
8

0
0
21
3

0
0
13
11

0
0
17
7

0
9
9
5

0
6
17
0

2
9
8
5

2
8
14
0

Sebaceous
gland infiltrate

Absent (0)
Mild/focal (0.11)
Moderate/multifocal (1.012)
Marked/diffuse (2.013)

Lymphocytic
infiltrate ued

Hyperkeratosis

Sweat gland
infiltrate

Intensity

Extent

Intensity

Extent

5
13
2
0

5
12
2
1

13
10
1
0

13
7
4
0

The number of dogs with an average histological score for each parameter. Intensity: absent (0), mild (0.011), moderate (1.012) and marked
(2.013).
Extent: absent (0), focal (0.011), multifocal (1.012) and diffuse (2.013). Ued = upper epidermis. Led = lower epidermis. Sd = superficial dermis.

mild, focal keratinocyte apoptosis and lymphocytic exocytosis in the upper epidermis and a
moderate-to-marked, multifocal lymphocytic exocytosis in the lower epidermis. In addition, there was
moderate-to-marked, multifocal superficial dermal
lymphocytic infiltrate. A lymphocytic interface mural
folliculitis was present above the infundibulum in all
dogs (where infundibula were present in the sections)
and below the infundibulum in 92% (21/23) of dogs
(Fig. 7). A lymphocytic sweat gland infiltrate was seen
in 46% (11/23) of dogs. Sebaceous glands were absent
from all skin biopsies evaluated in four dogs and
50% (25/50) of the total number of sections evaluated
(Fig. 8). In sections with sebaceous glands, a mild,
focal lymphocytic periglandular infiltrate was present
in 63% (12/20) of dogs. In one dog (case 10) progression of the disease over a 3-month period resulted in
complete absence of sebaceous glands from all skin
biopsy sections.

Direct immunofluorescence (IF)

Direct IF testing performed on paraffin-embedded
sections revealed the presence of in situ deposition of
IgG, IgM, IgA and C3 in the epidermal basement
membrane of 100% (19/19), 47% (9/19), 11% (2/19) and
5% (1/19) of dogs, respectively (see Table 5). Multifocal, continuous, fine deposition of IgG was recorded
in 61% (40/66), 35% (23/66) and 77% (50/66) of skin
biopsy sections, respectively (Fig. 9). IgG and IgM was
detected in the follicular basement membrane of 41%
(7/17) and 6% (1/17) of dogs, respectively (see Table 5).
Deposition of IgA and C3 in the follicular basement
membrane was not observed. In situ deposition of IgG
and IgM was observed in the sebaceous gland basement
membrane of the sections from one dog only (case 17).

Indirect immunofluorescence
Indirect IF testing on sections of normal canine hairedand salt-split-skin revealed the existence of circulating

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Characteristics of exfoliative cutaneous lupus erythematosus

Figure 6. Photomicrograph: canine epidermis. Lymphocyte-rich

interface dermatitis and superficial mononuclear dermatitis. H&E.
Bar = 35 m.

Figure 7. Photomicrograph: canine epidermis. Lymphocyte-rich

interface mural (infundibular) folliculitis with keratinocyte
apoptosis. H&E. Bar = 25 m.

antifollicular IgG antibodies at the 1 : 100 dilution in

the serum of 57% (4/7) of dogs (see Table 5 and Fig. 10).
In addition, antisebaceous gland IgG antibodies were
also detected at 1 : 101 : 100 dilution in these dogs.
Circulating antiepidermal basement membrane antibodies were not observed.

Immunohistochemistry
Immunohistochemical staining confirmed the predominance of CD3-bearing T lymphocytes in the lower
epidermis, superficial dermis, in the infundibulum of
hair follicles and around sweat glands in 21 dogs (see
Table 5 and Fig. 11). CD3-positive T lymphocytes infiltrated sebaceous glands and associated ducts in samples
collected from two dogs (see Table 5 and Fig. 12).

D ISCU SSION
This study confirms previously published reports
defining canine ECLE as a disease of young adult GSP.
While previous reports did not show a sex predilection,
it is interesting in this larger case series that the female
to male ratio was approximately 2:1. Scaling and alopecia

247

Figure 8. Photomicrograph: canine epidermis. Absence of

sebaceous glands. H&E. Bar = 600 m.

Figure 9. Photomicrograph: canine epidermis. IgG autoantibodies

are deposited along the epidermal basement membrane zone
(arrowheads). Direct immunofluorescence, anticanine IgGfluorescein with DAPI counterstain. Bar = 25 m.

are the most prominent clinical features. Lesions begin

on the muzzle, pinnae and dorsum and typically progress
to a generalized distribution.17 Follicular casts have
been reported previously1,4 and were present in several
affected dogs in this study. The casts represent an
accumulation of infundibulum stratum corneum
adhering to the hair shaft above the surface of the
follicular ostia and occur as primary lesions in vitamin
A responsive dermatoses, primary cornification defects
and sebaceous adenitis.911
The presence of intermittent pain and lameness in
several dogs in this study was a characteristic of ECLE
not consistently reported as a feature of the disease.17
In this study, dogs with lameness or pain presented
with a more severe phenotype of ECLE, with generalized scaling and crusting, depression and pyrexia.
Further diagnostic investigation including spinal radiographic evaluation, contrast myelogram, CSF analysis and multiple joint aspirates in a limited number of
cases failed to identify any concurrent musculoskeletal
abnormality. While a normal joint fluid aspirate would
eliminate the possibility of an active multiarticular
arthritis in these dogs, other musculoskeletal abnormalities including small joint arthralgia, myalgia,

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SL Bryden et al.

Table 5. Immunological Testing Results

Case #

DIF EBM

DIF FBM

DIF SBM

IIF EBM

IIF FOLL

IIF SEB

IHC

1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25

IgG, IgM
NT
NT
IgG
IgG
IgG
IgG, IgM
IgG
IgG
IgG
IgG
IgG
IgG, IgM
IgG
NT
NT
IgG, IgM
IgG, IgM
NT
IgG, IgM
NT
IgG, IgM
IgG, IgM, IgA
IgG, IgM, IgA, C3
IgG

NT
NT

IgG

IgG
NT
NT
IgG, IgM
IgG
NT

NT

IgG
IgG
IgG

NT
NT

NT
NT
IgG

NT

NT

neg 1 : 20
NT
NT
NT
NT
NT
NT
NT
NT
NT
NT
neg 1 : 20
neg 1 : 20
NT
NT
NT
NT
NT
Neg 1 : 10
Neg 1 : 10
Neg 1 : 10
NT
NT
Neg 1 : 10
NT

neg 1 : 20
NT
NT
NT
NT
NT
NT
NT
NT
NT
NT
Neg 1 : 20
Neg 1 : 20
NT
NT
NT
NT
NT
+1 : 100
+1 : 100
+1 : 100
NT
NT
+1 : 100
NT

neg 1 : 20
NT
NT
NT
NT
NT
NT
NT
NT
NT
NT
neg 1 : 20
neg 1 : 20
NT
NT
NT
NT
NT
+1 : 100
+1 : 50
+1 : 10
NT
NT
+1 : 100
NT

+
+
+
+
+
+
+
NT
+
+
+
+
+
+
+
+
+
+
+
+
NT
NT
+
+
NT

No deposition; NT Investigation not performed; DIF Direct Immunofluorescence, EBM epidermal basement membrane,
FBM follicular basement membrane, SBM sebaceous basement membrane; IIF Indirect Immunofluorescence, FOLL Hair follicle, SEB
Sebaceous gland; IHC Immunohistochemistry CD3+ T lymphocytes.

Figure 10. Photomicrograph: canine

epidermis. (a) Serum from a dog with ECLE
contains IgG that recognizes antigen(s)
along the sebaceous gland basement
membrane (arrowhead). (b) Normal canine
serum does contain such autoantibodies.
Indirect immunofluorescence using normal
canine haired skin, anticanine IgGfluorescein with DAPI counterstain.
Bar = 20 m.

myositis and tendonitis have been reported as an extracutaneous manifestation of systemic lupus erythematosus (SLE) in humans,13,14 and could account for the
undetectable source of pain in dogs with ECLE.
Haematological abnormalities of anaemia and
thrombocytopenia were also detected, in general, in a

limited number of dogs in this study with a more severe

clinical presentation of ECLE. Autoantibodies directed
against erythrocytes and platelets lead to haemolytic
anaemia and thrombocytopenia in canine SLE13,14 but
the significance of this finding in dogs with ECLE is
unknown. With the exception of one dog (case 14),

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Characteristics of exfoliative cutaneous lupus erythematosus

Figure 11. Photomicrograph: Canine Epidermis. T-lymphocytes

invade the lower epidermal layers. Immunohistochemistry CD3
staining. Bar = 100 m.

none of the dogs in this study had circulating ANA,

consistent with previous reports of ECLE.2 It is well
documented that ANA can be found in other canine
diseases as well as in normal dogs and are therefore not
specific for SLE.13,14
Microscopic examination of biopsy specimens
from affected ECLE dogs demonstrated a lymphocytic
interface dermatitis and mural folliculitis with an
absence of sebaceous glands consistent with previously
published reports.17,12 The histopathological findings

249

are, however, not pathognomonic for ECLE. Other

differential diagnoses may include discoid lupus
erythematosus (DLE), SLE, erythema multiforme (EM),
vesicular cutaneous lupus erythematosus (VCLE) of
the collie and Shetland sheepdog, and sebaceous
adenitis.1,12,15
Knowledge of the breed affected may be most helpful in differentiating ECLE from VCLE given the
strong breed predilection for collies and Shetland
sheepdogs to be affected with this latter disease.17,12,15
DLE lesions are usually restricted to the face and the
principal histopathological finding is a more intense
lichenoid interface band of dermal inflammation and
less marked hyperkeratosis than ECLE, although basal
cell vacuolar degeneration and blurring of the basement zone could be similar in both diseases.1,12
Clinically, the dermatological signs of canine SLE
are pleomorphic but can present as a generalized exfoliative dermatitis affecting the face, ears and distal
extremities.1,1214,16 The classic histopathological findings of canine SLE are similar to canine DLE with
more severe basal cell vacuolation and apoptosis and a
less intense lichenoid inflammation of the dermis.
The distinguishing histopathological features of ECLE
compared to classic SLE are the presence of moderate
to marked hyperkeratosis and, in some cases, the absence
of sebaceous glands. In addition, a clinical diagnosis of

Figure 12. Photomicrograph: canine

Epidermis. T-lymphocytes invade sebaceous
glands and sebaceous ducts.
Immunohistochemistry CD3 staining.
Bar = 150 m (inset: 100 m).
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250

SL Bryden et al.

SLE requires demonstration of multiple organ system

involvement with four of 11 criteria being satisfied
according to the classification system of the American
Rheumatism Association.14,16 Some cases of ECLE in
this study did present with concurrent haematological
and musculoskeletal abnormalities. However, these
occurred in a limited number of dogs and, with the
absence of nonerosive arthritis and detectable serum
ANA, would not satisfy the inclusion criteria required
for a diagnosis of SLE.
The most characteristic histopathological feature of
EM is the presence of apoptotic keratinocytes present
at all levels of the epidermis, accompanied by lymphocyte satellitosis.1,12 In ECLE, keratinocyte apoptosis appears to be principally confined to the basal cell
layers. However, individual necrosis of keratinocytes
was seen at all levels of the epidermis in approximately
30% of sections, making it difficult to distinguish
ECLE from EM reliably using this criterion. Hyperkeratosis is not a feature of acute EM, and athough
hyperkeratosis may occur with chronic EM it is rarely
marked, in contrast with the findings in ECLE.1,12 EM
is also typically negative on direct IF evaluation whereas
immunoglobulin deposition was identified at the basement membrane zone of all dogs with ECLE in this
study. Notwithstanding this, clinical differentiation
may be required: EM is characterized by an erythematous macular to papular eruption and it is uncommon
for the disease to occur in dogs of less than 1 year of
age compared with ECLE where adolescent GSP
dogs are affected with a predominantly exfoliative
dermatitis.1,12,17
Follicular casts or fronds associated with severe
adherent scaling and progressive alopecia are seen in a
predominantly dorsal distribution of some breeds with
sebaceous adenitis1,1012 and bears some resemblance
to the clinical presentation of dogs with ECLE. Furthermore, the diffuse absence of sebaceous glands and
unilateral peradnexal lymphocytic inflammation in the
site of the sebaceous glands in dogs with ECLE does
resemble the histological lesions of sebaceous adenitis.
An interface dermatitis and apoptosis are, however,
principal histological features of ECLE and not typical
histological features of sebaceous adenitis.1,12 Any disease involving primary or secondary destruction of
sebaceous glands could result in scaling and the histological absence of sebaceous glands thereby resembling
the syndrome of sebaceous adenitis
The presence of an interface dermatitis in dogs
with ECLE is suggestive of an underlying cytotoxic
T-cell-mediated pathogenesis. Similarly the presence of
CD3 bearing T lymphocytes in the epidermal, follicular and sebaceous gland basement membranes suggests
a major histocompatibility complex (MHC) restricted
cell mediated immunological reaction to antigen(s)
shared by these regions. Indirect immunostaining performed on normal and canine salt-split-skin revealed a
high titre of circulating IgG specific against follicular
basement membranes and sebaceous glands in more
than half the dogs evaluated in this study. This is in

contrast with previously published reports where no

circulating autoantibodies against sebaceous glands or
hair follicles were detected.2 This, in combination with
the presence of fixed tissue IgG antibodies present in
the epidermal basement membrane of all dogs in
this study, strongly suggests a combined cellular and
humoral immune response directed against basal cells
as the possible underlying pathogenetic mechanism in
ECLE. Inflammation of the basement membrane may
result in secondary destruction of sebaceous gland
germinative epithelium in the isthmus region, resulting
in the generation of sebaceous gland and follicular
autoantigens and the subsequent production of sebaceous gland and follicular specific IgG autoantibodies.
In general, the response to immunomodulatory
therapy for dogs with ECLE was poor, with short- to
medium-term clinical remission achieved with a combination of topical keratolytic and keratoplastic shampoo therapy and immunosuppressive treatment regimes
with prednisolone and/or azathioprine. More benign
immunomodulatory treatment combinations such as
oral tetracycline and niacinamide, oral essential fatty
acids and synthetic retinoids were not successful in
achieving remission or were frequently associated with
disease relapse. The majority of dogs in this study,
where a long-term or final outcome was recorded, were
euthanased either because of a failure to respond to, or
complications associated with immunomodulatory
therapy.
While the pedigree data are limited in this study, the
information available confirmed a shared ancestry in a
small number of affected dogs. The recognition of this
disease in littermates also strongly suggests a hereditary origin. The occurrence of ECLE in both male and
female dogs, the uncommon nature of the disease and
the relatedness among some of the affected dogs is
most suggestive of an autosomal recessive mode of
inheritance. However, a polygenic recessive mode
(wherein the dog must inherit several alleles in order to
have the phenotype) cannot be ruled out. It must be
emphasized that our data are very limited and pedigree
information from all affected individuals or breeding
studies would be required to confirm the exact mode of
inheritance.
In conclusion, this study demonstrates that canine
ECLE is typically a disease of young adult GSP with
an exfoliative dermatitis that presents variably with
lameness and pyrexia. The disease responds poorly to
immunosuppressive therapy and has a guarded prognosis. The classic histopathological features, although
not present in every affected dog, are a lymphocytic
interface dermatitis and mural folliculitis with secondary
loss of sebaceous glands. Furthermore, our findings
reveal the existence of a cellular and humoral immune
response directed against the epidermal basement
membrane, hair follicles and sebaceous glands of dogs
with ECLE. Additional studies are required to further
characterize the immunological pathogenesis of this
disease, with particular reference to the temporal relationship between the epidermal basement membrane

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252

Characteristics of exfoliative cutaneous lupus erythematosus

and adnexal immune response and the pathogenicity
and target of the antifollicular and antisebaceous
gland antibodies.

ACKN OWLEDGE ME NT S

7.

8.

The authors would like to thank the following

colleagues for providing case information and/or
histological slides: G. Burton, A. Cannon, E. Codner,
T. DeManuelle, G. Doering, R. Evans, K. Forstevedt,
A. Foster, C. Friberg, D. Gold, T. L. Gross, L. Jonas,
K. Kuhl, S. Shaw, M. Shipstone, S. Torres, C. Vitale
and C. Wraith. The authors would also like to thank R.
C. Tryon for assistance with the pedigree chart, A.
OHara for assistance with histopathology and J. Hood
for invaluable advice with the manuscript.

9.

10.

11.

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2. Olivry T, Luther PB, Dunston SM et al. Interface dermatitis and sebaceous adenitis in exfoliative cutaneous
lupus erythematosus (Lupoid Dermatosis) of German
Short-Haired Pointers. Proceedings of 15th AAVD/
ACVD Meeting 1999: 41 2.
3. Theaker AJ, Rest JR. Lupoid dermatosis in a German
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4. White SD, Gross TL. Hereditary Lupoid Dermatosis
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5. Vroom MW, Theaker MJ, Rest JR et al. Lupoid dermatosis in five German short-haired pointers. Veterinary
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Rsum Cette tude a caractris les donnes cliniques, histopathologiques et immunologiques de 25 cas de
lupus cutan exfoliatif, une dermatose exfoliative gnralise rare dcrite exclusivement chez les Braque allemands.
La maladie atteint des chiens jeunes adultes, et son incidence familiale suggre une origine hrditaire. Les lsions
sont caractrises par des squames et une alopcie affectant 100% (25/25) et 76% (19/25) des chiens respectivement. Des manchons pilaires taient nots chez 28% (7/25) des chiens. Le chanfrein, les pavillons auriculaires et
le dos taient typiquement atteints. Des lsions gnralises taient observes chez 52% (13/25) des chiens. Des
signes systmiques de douleur et de boiterie taient nots chez quelques chiens. Une anmie et une thrombocytopnie taient dtectes chez quelques chiens, atteints dun phnotype svre. Les lsions histopathologiques les
plus frquentes taient une hyperkratose et une dermatite dinterface lymphocytaire. Limmunomarquage direct
a montr un dpt dIgG dans la membrane basale de lpiderme et des follicules pileux dans 100% (19/19) et
41% (7/17) des cas respectivement. Des anticorps circulants antifolliculaires et anti glandes sbaces taient
dmontrs par immunofluorescence indirecte chez 57% (4/7) des chiens. Cette maladie rpond gnralement
mal aux traitements immunosuppresseurs et est de mauvais pronostic. Lorsquun suivi a t dcrit, 85% (10/12)
des chiens ont t euthanasis cause dune absence de rponse au traitement, ou aux complications de la
thrapeutique immunosuppressive. Deux chiens atteints taient en rmission avec des doses immunosuppressives
de prednisolone. Cette tude dmontre lexistence dune rponse cellulaire et humorale dirige contre la
membrane basale chez les chiens lupus cutan exfoliatif. Des tudes supplmentaires sont ncessaires pour
mieux caractriser la pathognie immunologique de cette maladie.
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252

SL Bryden et al.
Resumen En 25 perros se caracterizaron los procesos clnicos, histopatolgicos e inmunolgicos del lupus
eritematoso cutneo exfoliativo, una rara dermatitis exfoliativa que ocurre exclusivamente en Pointers alemanes
de pelo corto. La enfermedad afecta a jvenes adultos y su incidencia familiar sugiere claramente un origen
hereditario. Las lesiones se caracterizaron por descamacin y alopecia, afectando a un 100% (25/25) y a un 76%
(19/25) de los animales, respectivamente. Tambin se observaron cilindros foliculares en un 28% (7/25) de los
perros. Las zonas tpicamente afectadas incluyeron el hocico, orejas y el dorso. Un 52% de los perros presentaron
lesiones generalizadas (13/25). Varios perros se vieron afectados por signos sistmicos de dolor y cojera. Adems
en algunos perros se observ un fenotipo clnico ms severo con anemia y trombocitopenia. Las caractersticas
histopatolgicas ms comunes fueron hiperqueratosis y dermatitis linfoctica de la interfase. Mediante tincin
inmunolgica directa observamos depsitos de inmunoglobulina G en las membranes basles de la epidermis y
folculos pilosos en un 100% (19/19) y un 41% (7/17) de los perros, respectivamente. Por otro lado, mediante
tincin inmunolgica indirecta se detectaron anticuerpos circulantes del tipo IgG frente a las glndulas sebceas
y folculos pilosos en un 57% (4/7) de los perros. La enfermedad generalmente presenta una respuesta inadecuada
a la terapia inmunosupresora y un pronstico grave. En los casos en los que se sigui la evolucin clnica, un 85%
de los animales (10/12) fueron sacrificados debido a la pobre respuesta al tratamiento inmunomodulador o
debido a complicaciones derivadas del tratamiento. Dos de los perros afectados se encuentran actualmente en
remisin y se mantienen con dosis inmunomoduladoras de prednisolona. Este estudio demuestra la existencia
de una respuesta celular y humoral frente a la membrana basal de la epidermis en perros con lupus eritematoso
cutneo exfoliativo. Estudios complementarios seran necesarios para caracterizar la patognesis de la
enfermedad.
Zusammenfassung Klinische, histopathologische und immunologische Charakteristika von exfoliativem
kutanen Lupus erythematodes, einer seltenen generalisierten exfoliativen Dermatitis, die ausschliesslich beim
Deutsch Kurzhaar Vorstehhund vorkommt, wurden bei 25 Hunden beschrieben. Diese Krankheit betrifft junge
adulte Hunde und das familire Auftreten ist ein starker Hinweis auf einen erblichen Ursprung. Die Lsionen
waren charakterisiert durch Schuppenbildung und Haarausfall, die bei 100% (25/25) bzw. 76% (19/25) der Hunde
auftraten. Follikelkeratinmanschetten waren bei 28% (7/25) der Hunde vorhanden. Die Schnauze, die Ohrmuscheln
und der Rcken waren typischerweise betroffen. Generalisierte Hautlsionen wurden bei 52% (13/25) der
Hunde beschrieben. Etliche Hunde zeigten systemische Zeichen von Schmerz und Lahmheit. Anmie und
Thrombozytopenie wurde bei mehreren Hunden mit ausgeprgterem klinischen Phnotyp gefunden. The
hufigsten histopathologischen Befunde waren Hyperkeratose und eine lymphozytre Interface Dermatitis.
Direkte Immunfrbung zeigte IgG Ablagerung in der epidermalen und follikulren Basalmembran von 100%
(19/19) bzw. 41% (7/17) der Hunde. Zirkulierende IgG Antikrper, die gegen Haarfollikel und gegen Talgdrsen
gerichtet waren, wurden mit indirekter Immunfrbung bei 57% (4/7) der Hunde nachgewiesen. Diese Erkrankung
reagiert im allgemeinen schlecht auf immunsupprimierende Therapie und hat eine vorsichtige Prognose. 85%
(10/12) der Hunde, wo ein Endbericht vorlag, wurden euthanasiert entweder wegen fehlender Reaktion auf oder
wegen Komplikationen mit immunmodulierender Therapie. Zwei betroffene Hunde befinden sich in Remission
und werden mit immunmodulatorischen Dosen von Prednisolon dauerbehandelt. Diese Studie zeigt die Existenz
einer zellulren und humoralen Immunantwort, die gegen die epidermale Basalmembran bei Hunden mit
exfoliativem kutanen Lupus erythematodes gerichtet ist. Zustzliche Studien sind notwendig, um die
Immunpathogenese dieser Krankheit weiter zu charakterisieren.

2005 European Society of Veterinary Dermatology, Veterinary Dermatology, 16, 239252